Fibrogenic tumours Flashcards
calcifying aponeurotic fibroma plantar fibromatosis Desmoid tumour nodular facitiis malignant fibrous histocytoma Fibrosarcoma
What is a calicifying aponeurotic fibroma?
- A benign Fibrogenic mass that usually presents as a painless mass in hands/ feet
- In children young adults 3-30 yrs
How does a calicifying aponeurotic fibroma present?
- A painless growing mass in hands/feet
What is the imaging of calicifying aponeurotic fibroma?
- A faint mass with stippling
What is calicifying aponeurotic fibroma histology?
- fibrous tissue w central region of calcification and cartilage formation
What is the tx of calicifying aponeurotic fibroma?
Nonoperative
- Lesion often resolves with maturity
Operative
- Local excision
- recurrence up to 50%
What is planatr Fibromatosis?
- Aka Lederhosen disease
- Benign tumour of the foot plantar fascia that consists mainly of myofibroblasts and collagen
- similar process to Dupuytren’s in palmar fibromatosis
what is extra- abdominal desmoid tumour?
- A Fibrogenic lesio that is mostly locally invasive of all benign soft tissue tumours
- 15-40 yrs
- F>M
- location
- shoulder, chest wall/back, thigh
- >50% extra abdominal
- may have multiple lesions
- new lesion usually appears more proximal
- Genetics
- Patients with familial adenomatosis polyposis > risk of devloping extra- abdominal desmoid tumour
- Trisomy in chromosome 8 or 20
Name associated conditions of extra- abdominal desmoid tumour?
- Dupuytren’s disease
- Ledderhose disease
What is the prognosis of extra- abdominal desmoid tumour?
- High risk of recurrence
- highly unpredictable lesions
- occasional spontaneous regression
- NO risk of malignant change or mets unless hx of radiation
What are the symptoms of extra- abdominal desmoid tumour?
- Usually an enlarging Painless mass
- ‘rock hard’ on palpation
What is seen on imaging of extra- abdominal desmoid tumour?
- Xrays- not useful
- MRI
- best to dx
- low signal on T1
- Low to medium on T2
- Gadolinium enhances appearance
- may erode bone locally
what is seen on histology of extra- abdominal desmoid tumour
- marcoscopically
- gritty, white, poor capsulated
- Histology
- well differentiated fibroblasts
- uniform spindle cells w elongated nuclei
- abundant colagen
- Immunohistochemistry
- 100% positive for Estrogen receptor Beta
What is the tx of extra- abdominal desmoid tumour?
Non operatively
-
Low dose Chemotherapy only- Tamoxifen
- for inoperable lesions
- tamoxifen works via oestrogen receptor blockage
Operative
-
wide surgical resection w radiotherapy
- for symptomatic lesions/ recurrent lesions
- local reoccurance is common- reduced with radtx
- external beam radiotherapy up to 60Gy
What is nodular fascitis?
- A common reactive lesion that usually occurs in upper extremities
- you people 15-35
- half cases happen in Upper limbs
- painless/painful enlarging mass
- MRI- Inhomogenous mass, xrays normal
- Tx is operative- marginal excision
What is undifferentiated pleomorphic sarcoma?
- A **malignant fibrogenic tumour **
- 30-80 years
- similar to fibrosarcoma in presentation/tx
- PC- enlarging, painless mass
- symptoms start when mass >10 cm
- MRI- deep seated inhomogenous mass
- cartwheel pattern of spindle & histiocytic cells
- tx OPerative
- Wide local resection & adjuvant Radiotherapy
- add radiation if tumour >5cm
- 5000cGy neoadjuvant , 2000cGy adjuvant
What is fibrosarcoma of soft tissue?
- A malignant fibrogenic tumour
- pts 30-80 years
- same as Malignant Fibrous Histiocytoma of soft tissue
- PC usually painless enlarging mass (>10cm= symp)
- MRI- deep seated inhomogenous mass
- histology
- spindle shaped cells
- tissue organised in herringbone fashion
